A decade of Gottron’s papules, inverse Gottron’s papules and mechanic’s hand in anti-MDA5-associated interstitial lung disease with clinically amyopathic dermatomyositis

A 70-year-old man who was a former smoker presented to our hospital with fever and hemoptysis for 2 weeks. He had a rash in his bilateral upper eyelids and papules over his elbows and fingers for 10 years (Fig. 1A) but attributed it to his occupation, transport business. He had never experienced dyspnea before this admission. On admission, his body temperature was 37.4°C, and SpO₂ was 92% in room air. On physical examination, fine crackles were heard in the whole chest in addition to Gottron’s papules, inverse Gottron’s papules and mechanic’s hands (Fig. 1A–C). Chest computed tomography scan revealed subpleural superior ground-glass opacities in bilateral lower lobes, indicating interstitial lung disease (Fig. 1D). Laboratory examination showed creatinine kinase, 86 IU/L; lactate dehydrogenase, 400 IU/L; KL-6, 887 U/mL; anti-melanoma differentiation-associated protein 5 (anti-MDA5) antibody, positive (>150 indexes); anti-Jo-1 antibody, negative; antinuclear antibody, negative; negative dsDNA; anti-Sm antibody, negative; and anti-Scl-70 antibody, negative. Consequently, he was diagnosed with anti-MDA5-associated interstitial lung disease along with clinically amyopathic dermatomyositis (CADM). Despite optimal immunosuppressive and immunomodulatory therapy, his respiratory condition worsened gradually, and he died 2 months after the diagnosis.

Figure 1.

(A) Nailfold microvascular changes with cuticular overgrowth, periungual erythema and epithelial hemorrhage spots were present on the dorsum of the fingers (red arrows). Multiple hyperkeratotic and flat papules are present on the dorsum of the metacarpophalangeal and distal interphalangeal joints, indicating Gottron’s papules (white arrows). (B) Roughening and cracking of the skin are present on the sides of the fingers, indicating mechanic’s hand (red arrow). (C) Keratotic papules are present on the flexor side of the metacarpophalangeal joint, indicating inverse Gottron’s papules. (D) High-resolution computed tomography image showing bilateral honeycomb lung which is predominant in the right lower lung field with traction bronchiectasis (white arrow).

Bendewald et al. reported that CADM represented approximately 20% of all cases with dermatomyosis [1]. CADM might be complicated by acute/subacute interstitial pneumonia, especially among patients with anti-MDA5 antibody [2]. To the best of our knowledge, no comprehensive study is available on the speed of progression to CADM in patients with anti-MDA5 antibody. Although the precise duration of positivity of the antibodies is unclear, he had a 10-year history of unchanged cutaneous findings, which implies that inverse Gottron’s papules and mechanic’s hands can precede interstitial pneumonia for 10 years and that the first exacerbation of CADM could be fatal. Early detection and intervention might prevent the development of fatal interstitial pneumonia among patients with dermatomyositis.

FUNDING

The authors have no funding to report.

CONFLICT OF INTEREST STATEMENT

The authors have declared that no competing interests exist.

ETHICAL APPROVAL

This study was conducted upon approval by the Ethical Committee of Eiju General Hospital, an affiliate of Research Institute for Life Extension (2017–18).

CONSENT

Written informed consent was unobtainable because the patient was deceased.